Sunday, November 30, 2014

LA trip: MRI, LFT's and Keto, Dr. F, AGHD, friends and a documentary

Yeah, so that is a really long title, but I have a lot of updating to do on many subjects!

If you remember, Dr. F (my endocrinologist in LA) put me on Ketoconazole at my last appointment, gave me lab slips to check my liver enzymes after about 2 months on the medication, and gave me an order for a repeat MRI after about 3 months of treatment with keto.  You see, it appears that when keto messes with the feedback loop of ACTH and cortisol, it can sometimes spur the tumor to grow or produce more, making it more obvious.  I just say "makes it angry", so now you'll know what I mean by that.  But Keto also commonly causes liver damage, making the liver "angry" too.  (And this is the safest and least damaging of the few medications available for Cushing's; to my knowledge of the others, they involve multiple organs and can be really harsh).  I was on keto after my initial diagnosis in 2013 while I waited for surgery, and had no real problems.

Anyway, there are no 3T MRI machines in "Nowhere, Alaska", and no roads out.  The closest is Seattle and that place charges boatloads for their "magical" MRI's, making it cheaper by far for me to pay the added expense to fly to the Los Angeles area and have my MRI done there.  Plus, I like to.see Dr. F while I'm in town as in-person appointments just work so much better for me than phone ones or email ones.

So the second week of November, Britton and I flew down for my MRI and appointment.  We were lucky and were able to meet with a number of "Cushie's" while there (Friends I've made online, most I'd not met in person yet, all who have or had Cushing's Disease) which made the trip just amazing.  I'll talk more about that later and start with the medical stuff.

So, my MRI went well.  The machine in Torrance is SO much more comfortable than the GE one I was in at MDA, and this one didn't try to burn my arms at the end, though their headphone music system wasn't working so I had to spend an hour with my thoughts and some really loud noises with nothing to look at (no, falling asleep isn't an option for me; I wish!). 

When we met with friends at the hotel that afternoon, we put my disc in their computer and sent a drop-box copy to another "cushie" friend who loves to read MRI's as a hobby (not an MD, but she's pretty dang good).  There were new signs of empty sella, which means my pituitary is flattening, and there is (Cerebro-Spinal Fluid) CSF in my sella.  Empty sella syndrome tends to lead to hypopituitarism.  So far I'd been okay and my pituitary was still functioning normally...though that isn't to say it won't lose function over time.  There is no way to know, we just have to watch symptoms and test routinely as time goes on to see if things worsen.

There were two other things noticed...a possible new tumor on my MRI, when compared to my old pre-op MRI (different spot but same side of pituitary), and some crazy stuff going on in my sinus that looks like it is full of bubbles (on CT in August it looked like a solid mass about 2cm by 2.5cm, that the ENT's thought was scar tissue).  None of us had seen that before, and my friend called it a "train wreck."

At my appointment, we discussed my liver function tests (LFT's), which I had drawn the week before and had come back significantly elevated (like 5 times the high end of the normal range), and I was taken off of ketoconazole.  Luckily I'd swung into a low cortisol cycle the week before and had stopped taking it because it was making me swing too low and I hadn't restarted yet. 

Not surprisingly, Dr. F viewed my MRI and made the same statements my friend had (aside from the sinus one).  He suggested I send my MRI on to Dr. McC (neurosurgeon in Houston) to get his opinion on whether that was a target (ie, tumor).  The radiologist later called it "changes from surgery", but he also admitted to missing the original tumor on my very first MRI, so I'm waiting for an expert to weigh in on that.  Dr. F also wanted to retest my thyroid and growth hormone (GH), because those are usually the first two hormones to be affected by pituitary damage (the empty sella).

My thyroid is still happy, but my IGF-1 (the test they run to check GH) had dropped by half and was now well below the normal range for my age and gender.  I'm not sure if my low IGF-1 was affected by my liver damage, so we are retesting my liver weekly and then will go from there.  If that IGF-1 stays low, I will need a GH stim test to prove my pituitary isn't capable of producing enough (for the insurance company who HATES to pay for this expensive medication --and it IS expensive) and then I will go on daily injections for life to replace the hormone my body can no longer make.  (FYI, a stim test is where they force your body, through medication, to make as much growth hormone as it can and test at exact time intervals to see your body's response.)

Here is where you say, "Growth hormone?!  You're 33!  Surely you've stopped growing by now?  Why would you need daily GH?"  Well, here's why: Adult Growth Hormone Deficiency (AGHD) causes all kinds of problems.  It's like cortisol in that it does a specific job, and that's all we tend to think about, but when it is out of whack, all KINDS of body systems suffer.  Just to name a few, it leads to abnormally high cholesterol and thus cardiac/artery issues.  It leads to thinning bones and muscle loss.  It leads to accumulation of visceral fat.  The list goes on, and it is not pretty.  Combine that with the big list of what Cushing's does, and some of those equate to a double whammy.  I'm 33, I certainly don't want a heart attack or osteoporosis already!  The side effects of taking too much are swelling or pain in the hands...when that hits, you can simply lower your dose.  Not hard to balance those risks/benefits, eh?  And just in case you're wondering, it is taken for life because pituitary's aren't known to recover.

Livers, though, can.  I've since tested on the 20th and 26th, and from the 7th to the 26th, my LFT's dropped by half!  Half of that in those last 6 days!  It looks like the momentum of my liver's recovery with the medication out of my system is improving and I'll be within normal range in no time.  I will test those again this next week and see how I'm doing (Dr. F's suggestion), and then we can progress with testing for GH and be certain the results will be accurate. 

If I need a stim, it cannot be done locally (I've called everyone including the hospital), so I've already contacted Swedish and would have it done by Dr. Yuen there.  He's an endocrinologist on Dr. F's recommended endo's list who used to work out of OHSU in Oregon and recently switched to working at Swedish's pituitary center.  YAY!  His scheduler said all I would need is the order from Dr. F and/or a referral sent to them and I could schedule my GH stim with about a week wait between making the appointment and having the test (Minus the winter holiday weeks).  Bonus: He's done MANY studies on growth hormone. 

And something else that is awesome?  Both a Cushie friend and an AGHD/hypo-pit friend (that I met at the Magic Foundation's adult conference this April) live in the general area and have offered me a place to stay!  Obviously this calls for two trips, right?  (Oh, I wish!!!)

Hmm...what else?  Oh, friends and the documentary.  Well, that part was just awesome, but this post is already long and wordy so I probably should save it for a future post.  CLIFF HANGER!

ETA: I forgot to mention that I am also retesting for Cushing's, to see if my testing is more definitive for a repeat diagnosis (and moving on to repeat surgery).  It'll take me some time,'s a great many tests that must be done while I'm in a high cycle.  Add to that that neither lab in town can do all of the tests (each can do some of my list, meaning all get hit but I have to juggle between them) and they have different drop off restrictions for time of day and days of the week.  Thanksgiving didn't help that, either.  ;)